Table 4

Patients with Usher syndrome 1—clinical information

No Age/ sex Age/diag deafness Audio gram Vestibular function Age diag RP (years) Age visual symptom (years) VA better eye ERG
( mV)
Cat Field (°) Pigment degen
10* 47/MInfantNRneg21153/60extno10Extensive
Atrophic maculae
11* 45/FInfantNRneg2222PLextno<5Extensive
Atrophic maculae
18* 23/F2 yearsNRneg23216/9extno20Extensive
22* 25/FInfantNRneg17126/12extno10Moderate
24* 46/F2 yearsNRneg18246/12extbilat pseudo phakos5Extensive
Macular pigment
25* 36/MInfantNRNP8133/60extno<5Extensive
2734/F2 yearsNRNP15126/9extno10Extensive
2821/MInfantNRNP18156/9<75noconstrictedModerate
32* 23/M2 yearsNRneg12126/6extnoconstrictedModerate
36* 39/FInfantNRneg34186/12extearly PSLO20Extensive
  • RP = retinitis pigmentosa; NR = non-recordable; NP = not performed; PSLO = posterior subcapsular lens opacities; ext = extinguished; Cat = cataract; diag = diagnosis; pigment degen = pigmentary degeneration; *clinically reassessed.